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EPILEPTIC SYNDROME IN CHILDHOOD

BENIGN EPILEPSY
WITH BENIGN OCCIPITAL
CENTROTEMPORAL EPILEPSY
SPIKES

PANAYIOTOPOULOS
SYNDROME
BENIGN PARTIAL EPILEPSY OF CHILDHOOD WITH
CENTROTEMPORAL SPIKES (BECTS)
 ≈ Benign Rolandic Epilepsy (BRE)
 ~ 15 % of childhood epilepsy
 Best prognosis of all epilepsies*
 Age onset 4-10 years
 Commonest: is sleep, simple partial (face, tongue), secondary generalized (not uncommon)**
 Diagnosis: normal neurologic exam, and typical EEG (centrotemporal spikes, anterior-posterior dipole)
 Treatment is optional
 Frequent seizures, secondary GTCSs or comorbid conditions may need medication
 Virtually every AED is efficacious, bur some may significantly reduce GTCSs without reduction of focal seizures.
 Empirically, carbamazepine is preferred. Exceptional cases  may worsen seizures.
 Recent studies found levetiracetam  highly effective
 Clobazam single dose at bedtime
BENIGN OCCIPITAL EPILEPSY
Everything that benign Rolandic is not
No typical seizure type and no clearly defined clinical course
May be of any type, although diagnosis most palatable if there is at least a visual
aura
EEG shows occipital spike-waves (in chains) and block with eye opening*
PANAYIOTOPOULOS SYNDROME (PS)
 Benign age-related focal seizure
 Early and mid childhood
 Predominantly autonomic symptoms*
 EEG shows shifting and/or multiple foci (often with occipital predominance)
 PS is not ‘occipital’ epilepsy**
 Prophylactic treatment with AED may not be needed for most patients
MANAGEMENT OF BENIGN CHILDHOOD FOCAL SEIZURES

Lengthy convulsive seizures are a medical emergency: rectal or buccal


benzodiazepine

Appropriate aducation and emotional support should be provided

Acute management of a child with prolonged seizures:


• CONTROL of the seizure
• Autonomic status epilepticus – avoid aggressive treatment  risk of iatrogenic complications
(cardiorespiratory arrest)
• Prolonged convulsive or SE  SE guideline
MANAGEMENT OF BENIGN CHILDHOOD FOCAL SEIZURES

Prophylactic AED treatment

• Continuous treatment is not usually recommended


• Benefit risk consideration
• Recurrent seizures and/or parental anxiety  small doses od AED
• Consideration:
• Most have excellent prognosis
• Remission is expected in all patients by age of 15 or 16
• Possibility of future epilepsy is most unlikely*
• No evidence that long-term prognosis is worse in untreated children
• Some children become frightened, even by simple focal seizure, and some parents cannot cope
with the possibility od another fit
• Persistence and frequency of EEG functional spikes do not predict clinical severity, frequency or
degree of liability to reccurent seizures
MANAGEMENT OF BENIGN CHILDHOOD FOCAL SEIZURES

Continuous prophylaxis, daily monotherapy, any AED that has proven efficacy in focal seizures and minimal
AE in children
2006 ILAE treatment guidelines  no AED had level A or level B efficacy and effectiveness evidence as
initial monotherapy in BRE

USA  most authorities prefer CBZ; Europe  valproate

CBZ may exaggerate seizures in a minorityof children with BCSSS (incl. PS) and valproate associated with
significant ADRs.
Sulthiame (available only in a few countries)  excellent drug for the treatment of BRE with EEG
normalisation.***

recommended newer AED: levetiracetam, oxcarbazepine and gabapentin.

Lamotrigine  also used, though have been associated in a few reports with seizure exacerbation and
cognitive deterioration in BRE.
CHILDHOOD ABSENCE EPILEPSY

OTHER ABSENCE EPILEPSIES

CRYPTOGENIC PARTIAL SEIZURES STARTING IN CHILDHOOD

GENERALIZED EPILEPSY WITH FEBRILE SEIZURES PLUS (GEFS+)


CHILDHOOD ABSENCE EPILEPSY
ONSET age 4-10 y.o.
Neurologically normal
Attacks are frequent and last 5-15 s
Ictal and interictal EEG shows “classic” 3-Hz spike-and-wave with normal background
Treatment: ethosuximide (ESM), valproic acid (VPA), or lamotrigine (LTG)
Medication may be unsuccessfull
Why??
1. Medication may be unsuccessful
2. Often associated with cognitive/learning problems
3. Evolve to the life-long disorder of JME
4. Social outcome of young adults who had CAE is unfavourable
5. CAE may have significant effects on the child’s development
OTHER ABSENCE EPILEPSY
Micturitional absence Myoclonic absence Absence with eyelid
myoclonia
• Powerful bladder • During seizure, besides • Marked eyelid and upper
detrusser contraction with absence manifestation, facial jerking during the
urination also some jerking motions, seizures
• Very frequent, 3Hz • Autosomal dominant
• very resistant to • Seizures are frequent, but • Typically very resistant to
medication do not fall conventional treatment
• Very socially problematic • No post ictal confusion
– constantly wet • Most are mentally
• Significant remission rate* handicappped
• Very resistant to treatment
• May “die down” but other
generalized seizure type
continue
CRYPTOGENIC PARTIAL SEIZURES STARTING IN CHILDHOOD
1. Many otherwise normal children have partial epilepsy  no known cause,
proceeds with a benign course
2. Not easy to characterize accurately –– benign clinical course OR persistent
epilepsy
3. The cause of epilepsy was truly unkown
4. Normal intelligence and neurologic exam
5. ~ 50% have remarkable benign disorder
GENERALIZED EPILEPSY WITH FEBRILE SEIZURES
PLUS (GEFS+)
1. Autosomal dominant
2. Caused by a defect in the neuronal voltage-gated sodium channel
3. In its simplest form  ordinary febrile seizures that continue to an older age than
usual
4. In adolescence there are GTCS with eventual remission
5. ~30% of those carrying the gene have other epilepsy syndrome with different
seizure types incl. absence, myoclonus, and akinetic
CONTINUOUS SPIKE-WAVE IN SLOW SLEEP (CSWS)

LANDAU-KLEFFNER SYNDROME

LENNOX-GASTAUT SYNDROME

MYOCLONIC-ASTATIC EPILEPSY